CLINICAL-FEATURES OF THE HEMORRHAGE TYPE MOYAMOYA DISEASE BASED ON 31CASES

We evaluated and analysed our own 31 cases of the haemorrhagic type of moyamoya disease to clarify the clinical features of this disease. Th e cases were divided into three groups. Group A consisted of 12 cases with aneurysms. Aneurysms on the circle of Willis were treated as ordi nary saccular aneurysms. Group B consisted of 14 cases with intracereb ral haemorrhage (ICH) without aneurysms. These were managed almost as spontaneous ICH. Group C consisted of 5 cases with intraventricular ha emorrhage (IVH) without aneurysm or ICH. Twenty-two surgical procedure s for aneurysms. ICH and IVH were done in 19 cases (62%). Nine teen pr ocedures for preventing future strokes were undertaken in 11 cases (35 %). The overall initial outcome was excellent in 12 cases (39%). good in 7 cases (23%). pool in 7 cases (23%). and death in 5 cases (15%). D uring the follow-up period (mean: 6.5 years. rebleeding occurred in tw o cases (8%), and ischaemic attacks in two cases (8%). The rate of reb leeding or ischaemic attacks was 1.19% per patient-year during the fol low-up period. There was no ischaemic or rebleeding episode in cases t reated by STA-MCA bypass with encephalomyosynagiosis (EMS) during the follow-up period. Management of the primary haemorrhage should be acco rding to the clinical condition, type of haemorrhage. and sourer of ha emorrhage. When the patient needs to undergo revascularization surgery to prevent future strokes, we recommend STA-MCA bypass with EMS inste ad of encephaloduro-arteriosynangiosis (EDAS).